photo rounds
A newborn with a skin lesion
on the back
A
24-year-old woman without
a family history of congenital
anomalies delivered her first
baby after a minimally complicated prenatal course. Poor weight gain led to an
ultrasound at 33 weeks gestation; this
test and others at 13 weeks to determine
gestational age and at 20 weeks to evaluate for anatomic abnormalities all had
®
normal results.
Prenatal screenings included a normal alpha feta-protein level. Review of
the mother’s medical records suggested
compliance with routine prenatal care
and vitamins; however, she smoked cigarettes during the pregnancy and occasionally took a pill containing butalbital, acetaminophen, and caffeine for migraine
Timothy J. Benton, MD,
Rupinder Cheema, MD,
and Jason G. Nirgiotis, MD
School of Medicine, Texas
Tech University Health Sciences
Center at Amarillo
headaches. She did not have gestational
diabetes, but late in the pregnancy she
developed hypertension, necessitating
induction of labor.
She delivered (via cesarean section)
a full-term baby girl weighing 6 pounds,
9 ounces. The infant appeared healthy,
with Apgar scores of 8 and 9 and a normal physical exam, except for a 2.5-cm
appendage overlying the spine (Figures
1 and 2). The 2-day nursery stay was
that of a normal neonate, without complications. The pediatric surgeon who
was consulted arranged for outpatient
follow-up after hospital dismissal.
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figure 1
z
What is your diagnosis?
figure 2
Skin lesion
Close-up
correspondence
Timothy J. Benton, MD
Assistant Professor, Associate
Residency Program Director,
Texas Tech University Health
Sciences Center at Amarillo,
School of Medicine, Department
of Family and Community Medicine,
1400 Wallace Boulevard, Amarillo,
Texas 79106.
The small appendage was in the middle of the infant’s
lower back.
www.jfponline.com
A close-up of the lesion, which contained primarily fatty
tissue.
E-mail:
[email protected]
vol 56, No 1 / january 2007
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photo rounds
Diagnosis
fast track
When a tail-like
structure is seen
on a neonate,
consider the
possibility of
occult spinal
dysraphism before
removing it
E2
OSD at birth.4 Although 3% to 8% of
Pseudotail
newborns with a skin lesion over the
Inspection of the lesion at the time of ex- spine have OSD,5 50% to 90% of pacision revealed fatty tissue within its core, tients with OSD have a skin marker.5–7
consistent with the diagnosis of pseudo- Of 2010 newborns in 1 study, 144 had
tail. Ultrasound of the spine performed a midline cutaneous lesion, and 5.5% of
before hospital discharge was normal.
these had an abnormal ultrasound suggesting OSD.8 Additionally, the presence
of more than 1 lesion increases the probz Tails and pseudotails
ability of finding OSD.5,9,10
Tails and pseudotails appear similar on
You should look for underlying OSD
physical exam and are differentiated in newborns with a suggestive skin lehistologically. True tails—remnants of sion, since the embryologic origin of the
the embryologic tail—contain a core of spinal cord and the skin overlying the
muscle fibers, nerves, and blood vessels, spine are the same: the ectoderm layer
whereas pseudotails contain primarily of cells within the trilaminar (endoderm,
fatty tissue.1–3 From a practical and clini- mesoderm, ectoderm) early embryo gives
cal point of view, you do not need to dif- rise to both structures. Midline cutaneous
ferentiate tails and pseudotails as they lesions with high likelihood for OSD inboth can be associated with skin-covered clude hypertrichosis (hairy patch), atypicongenital anomalies of the spine, or cal dimple (a dimple >5 mm diameter or
occult spinal dysraphism (OSD).
>2.5 cm above the anus), hemangioma,
Although tail-like structures rarely lipoma, aplasia cutis, dermoid cyst or sioccur, when they are present you should nus, skin tags, tails, and pseudotails.3
consider OSD.3 Two noteworthy literature reviews support this. The first identified 33 tails and pseudotails, of which Evaluation
10 had OSD.1 An analysis of more recent The neonate with a midline
cases (1960 through 1997) demonstrated cutaneous lesion
OSD in 29 of 59 cases with lesions de- The incidence of OSD appears low in
scribed as tails.2 A higher incidence of newborns with midline cutaneous lesions,
OSD occurred between 1980 and 1997 but irreversible orthopedic, urologic, and
in this series. The authors attribute the neurologic problems from OSD may ochigher incidence to the advent of com- cur later in life. These complications can
puted tomography and magnetic reso- be prevented by early surgical intervennance imaging (MRI), thereby stressing tion; therefore, it is critical that you do
the importance of spine imaging with the not miss a diagnosis of OSD and pursue
presence of any caudal appendage resem- spine imaging.
bling a tail.
The definitive test, an MRI,3,6,9 usually requires sedation of infants,11 making
high-resolution ultrasound the recomz Epidemiology of occult
mended choice for initial screening.3–10
spinal dysraphism
Ultrasound is not invasive and may allow
The true incidence of OSD is not known. for testing in the hospital nursery. After
Congenital anomalies of the central ner- age 3 months, when ossification of the
vous system, second only to birth defects posterior spine limits visualization of the
of the cardiovascular system, are report- spinal cord by ultrasound, consider an
ed on birth certificates, but OSD may MRI first. If the ultrasound demonstrates
go unrecognized in the neonatal period. OSD or is inconclusive, follow with an
Frequently a benign-appearing midline MRI for a more specific diagnosis.
cutaneous lesion is the only evidence of
Indications for an ultrasound of the
vol 56, No 1 / january 2007 The Journal of Family Practice
newborn’s spine include any one of the
following: an abnormal antenatal ultrasound; a midline cutaneous lesion other
than a simple dimple; presence of other
OSD related anomalies; or an abnormal
neurologic exam.5 The authors of this
protocol retrospectively applied it to 223
infants who had an ultrasound, and it reduced the number of tests by 50% without missing a single case of OSD.
If you are caring for a newborn infant
you should resist the temptation to simply remove a midline cutaneous lesion,
including a pseudotail, in the nursery
without first searching for OSD. If OSD
is present, neurosurgical consultation
should be obtained as soon as possible.
The optimal time for surgical intervention is within the first 4 months of life.4
z
s
A newborn with a skin lesion on the back
review of 54 cases. Arch Dermatol 2004; 140:1109–
1115.
10.Kriss VM, Desai NS. Occult spinal dysraphism in
neonates: assessment of high-risk cutaneous stigmata on sonography. AJR Am J Roentgenol 1998;
171:1687–1692.
11.Keengwe IN, Hegde S, Dearlove O, Wilson B, Yates
RW, Sharples A. Structured sedation programme
for magnetic resonance imaging examination in
children. Anaesthesia 1999; 54:1069–1072.
Photographs contributed by Jason G. Nirgiotis, MD.
Patient outcome
The infant’s pseudotail was removed
without consequence and postoperatively the patient has thrived.
c o n f l i c t o f i n t e re s t
The authors do not have any conflict of interest related
to this manuscript.
fast track
High-resolution
ultrasound is
recommended
for the initial
screening of OSD
re f e re n c e s
1.Dao AH, Netsky MG. Human tails and pseudotails.
Hum Pathol 1984; 56:339–340.
2.Lu FL, Wang PJ, Teng RJ, Yau KI. The human tail.
Pediatr Neurol 1998; 19:230–233.
3.Drolet B. Cutaneous signs of neural tube dysraphism. Pediatr Clin North Am 2000; 47:813–823.
4.Kriss VM, Kriss TC, Desai NS, Warf BC. Occult
spinal dysraphism in the infant. Clin Pediatr 1995;
34:650–654.
5.Robinson AJ, Russell S, Rimmer S. The value of
ultrasonic examination of the lumbar spine in infants with specific reference to cutaneous markers of occult spinal dysraphism. Clin Radiol 2005;
60:72–77.
6.McAtee-Smith J, Hebert AA, Rapini RP, Goldberg
NS. Skin lesions of the spinal axis and spinal dysraphism. Fifteen cases and a review of the literature.
Arch Pediatr Adolesc Med 1994; 148:740–748.
7.Dick EA, Patel K, Owens CM, De Bruyn R. Spinal
ultrasound in infants. Br J Radiol 2002; 75:384–
392.
8.Henriques JG, Pianetti G, Henriques KS, Costa P,
Gusmao S. Minor skin lesions as markers of occult
spinal dysraphisms—prospective study. Surg Neurol 2005; 63(Suppl 1):S8–S12.
9.Guggisberg D, Hadj-Rabia S, Viney C, et al. Skin
markers of occult spinal dysraphism in children: a
www.jfponline.com
vol 56, No 1 / january 2007
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